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childhood epilepsy

Rebecca Korth, Renate Schepker
Brain X-radiation for Childhood Epilepsy, Hydrocephalus or Mental Retardation? Research at Tuebingen University, 1940-1946 We reconstructed 65 cases out of a series of "experimental" X-ray-therapy by chart review and reanalysis of publications from a contextual historical perspective. The research procedures in the context of NS-pressure for effectiveness soon dismissed structured scientific procedures and surrendered own standards, whereas radiation impact did not transgress the contemporary guidelines...
September 2017: Praxis der Kinderpsychologie und Kinderpsychiatrie
Ana Catarina Franco, Olympia Kremmyda, Jan Rémi, Soheyl Noachtar
OBJECTIVE: Positive interictal epileptiform discharges (IEDs) are rarely recorded from surface EEG, due to the orientation of the cortex and its neurons. Their frequency and significance in adults is unknown, and has only been studied as a phenomenon of the neonatal period and childhood. We aimed to evaluate the frequency and characteristics of positive epileptiform discharges in a large cohort of patients. METHODS: We retrospectively reviewed 24,178 reports from 18,060 patients of non-invasively recorded EEGs for various indications...
February 8, 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
Katharine Bailey, Nancie Im-Bolter
Epilepsy is the number one neurological disorder in children in western society. Childhood epilepsy is highly comorbid with psychopathology. Although neurological and biological factors may partially explain the increased risk of psychopathology in children with epilepsy, social contextual factors are also important to understanding development of psychopathology in children with epilepsy. The current paper examines the development of children with epilepsy utilizing Bronfenbrenner's micro-, meso-, exo-, and macrosystem social contexts...
March 7, 2018: Seizure: the Journal of the British Epilepsy Association
Davide Caputo, Marina Trivisano, Federico Vigevano, Lucia Fusco
CHD2 gene has been described in association with different types of childhood myoclonic epilepsy and is emerging as a gene involved in photosensitivity alone or combined with epilepsy. Recent studies suggest that CHD2 could be responsible for a proper phenotype characterized by infantile-onset generalized epilepsy, intellectual disability, and photosensitivity and in particular with self-induced seizures. We report the case of a child with CHD2 mutation and mild developmental impairment that since the age of 3 years started with myoclonic seizures apparently well responding to antiepileptic drugs and that subsequently developed intractable self-induced seizures...
March 3, 2018: Seizure: the Journal of the British Epilepsy Association
David Neubauer, Mirjana Perković Benedik, Damjan Osredkar
PURPOSE: Refractory epilepsies in children present a major burden for patients and their families. Cannabidiol (CBD) has been suggested as a potential treatment for refractory epilepsies. The aim of this study was to evaluate the effectiveness of add-on therapy with CBD for the treatment of refractory childhood epilepsies. METHOD: Patients with childhood-onset refractory epilepsy, treated at the tertiary epilepsy center of the University Children's Hospital Ljubljana, Slovenia, were included in the study...
March 8, 2018: Epilepsy & Behavior: E&B
Chamara Arachchighe Lahiru Weerasinghe, Bich-Hong Thi Bui, Thu Thi Vu, Hong-Loan Thi Nguyen, Bao-Khanh Phung, Van-Minh Nguyen, Van-Anh Pham, Vu-Hung Cao, Tuan-Nghia Phan
Leigh syndrome is a rare inherited, heterogeneous and progressive neurometabolic disorder that is mainly caused by specific mutations in nuclear DNA (nDNA) or mitochondrial DNA (mtDNA). The present study reported a case of childhood Leigh syndrome with a point mutation at bp 8,993 in the mitochondrial ATPase6 gene. A 21‑month‑old male child had developed epilepsy, muscular weakness and vomiting, which was accompanied by high fever. Magnetic resonance imaging indicated typical characteristics of Leigh syndrome, including a symmetric abnormal signal in the dorsal medulla oblongata and Sylvian fissure enlargement in association with an abnormal signal in the periventricular white matter and in the putamina and caudate heads...
March 1, 2018: Molecular Medicine Reports
Trishna Kantamneni, Lileth Mondok, Sumit Parikh
Movement disorders in the pediatric age group are largely of the hyperkinetic type. Metal ion accumulation in the central nervous system presents predominantly with movement disorders and over time leads to psychomotor decline. Abnormalities in monoamine and amino acidergic neurotransmitter metabolism present in individuals with a combination of abnormal movements, epilepsy, and cognitive and motor delay. Detailed clinical history, careful examination, appropriate diagnostic work-up with metabolic screening, cerebrospinal fluid neurotransmitters, and targeted genetic testing help with accurate diagnosis and appropriate treatment...
April 2018: Pediatric Clinics of North America
Seyhan Hidiroglu, Nimet Emel Lüleci, Melda Karavus, Ozlem Tanriover, Elif Samiye Bayar, Ahmet Karavus
OBJECTIVE: To assess the awareness of childhood autism among physicians undergoing residency training in various disciplines. METHODS: This cross-sectional study was conducted at a research and training hospital in Istanbul, Turkey, in February 2013 and comprised physicians undergoing residency training in various disciplines. Data was collected through a self-administered questionnaire. Questions about "awareness on autism" were prepared in the light of "Knowledge about Childhood Autism among Health Workers questionnaire...
February 2018: JPMA. the Journal of the Pakistan Medical Association
Karen S Ho, Leah M Markham, Hope Twede, Amanda Lortz, Lenora M Olson, Xiaoming Sheng, Cindy Weng, E Robert Wassman, Tara Newcomb, E Robert Wassman, John C Carey, Agatino Battaglia
Seizures are present in over 90% of infants and children with Wolf-Hirschhorn syndrome (WHS). When present, they significantly affect quality of life. The goal of this study was to use caregiver reports to describe the comparative efficacies of commonly used antiepileptic medications in a large population of individuals with WHS. A web-based, confidential caregiver survey was developed to capture seizure semiology and a chronologic record of seizure treatments as well as responses to each treatment. Adverse events for each drug were also cataloged...
February 22, 2018: Epilepsy & Behavior: E&B
Nicolas Gaspard, Lawrence J Hirsch, Claudine Sculier, Tobias Loddenkemper, Andreas van Baalen, Judette Lancrenon, Michel Emmery, Nicola Specchio, Raquel Farias-Moeller, Nora Wong, Rima Nabbout
We report the proceedings of the First International new-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES) Symposium. To promote awareness of this condition and foster research efforts, we conveyed the First International new-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES) Symposium. The conference was supported by The NORSE Institute ( This article summarizes the discussions that were held during the Symposium and presents our strategy to unravel the cause of these disorders and to improve patient care...
February 24, 2018: Epilepsia
Moritz Tacke, Ingo Borggraefe, Lucia Gerstl, Florian Heinen, Katharina Vill, Michaela Bonfert, Thomas Bast, Bernd Axel Neubauer, Friedrich Baumeister, Martina Baethmann, Karl Bentele, Christian Blank, Harald M Blank, Harald Bode, Friedrich Bosch, Ulrich Brandl, Knut Brockmann, Peter Dahlem, Jan-Peter Ernst, Evemarie Feldmann, Andreas Fiedler, Michael Gerigk, Soeren Heß, Christiane Hikel, Hans-Georg Hoffmann, Matthias Kieslich, Joerg Klepper, Gerhard Kluger, Hartmut Koch, Walter Koch, Rudolf Korinthenberg, Ilona Krois, Hermann Kühne, Gerhard Kurlemann, Michaela Mandl, Ulrike Mause, Peter Navratil, Joachim Opp, Johann Penzien, Viola Prietsch, Axel Quattländer, Dietz Rating, Ulrike Schara, Mohammed G Shamdeen, Andreas Sprinz, Hildegard Wendker-Magrabi, Ulrich Stephani, Hiltrud Muhle, Hans-Michael Straßburg, Bärbel Töpke, Regina Trollmann, Elisabeth Tuschen-Hofstätter, Stephan Waltz, Gabriele Weber, Frank U Wien, Markus Wolff, Tilman Polster, Hedwig Freitag, Ötzcam Sönmez, Klaus Reinhardt, Marion Traus, Zeecam Hoovey
PURPOSE: BECTS (benign childhood epilepsy with centrotemporal spikes) is associated with characteristic EEG findings. This study examines the influence of anti-convulsive treatment on the EEG. METHODS: In a randomized controlled trial including 43 children with BECTS, EEGs were performed prior to treatment with either Sulthiame or Levetiracetam as well as three times under treatment. Using the spike-wave-index, the degree of EEG pathology was quantified. The EEG before and after initiation of treatment was analyzed...
February 3, 2018: Seizure: the Journal of the British Epilepsy Association
Lin Jiang, Tijiang Zhang, Fajin Lv, Shiguang Li, Heng Liu, Zhiwei Zhang, Tianyou Luo
Benign childhood epilepsy with centrotemporal spikes (BECTS) is associated with cognitive and language problems. According to recent studies, disruptions in brain structure and function in children with BECTS are beyond a Rolandic focus, suggesting atypical cortical development. However, previous studies utilizing surface-based metrics (e.g., cortical gyrification) and their structural covariance networks at high resolution in children with BECTS are limited. Twenty-six children with BECTS (15 males/11 females; 10...
2018: Frontiers in Neurology
Mohit Dubey, Eelke Brouwers, Eline M C Hamilton, Oliver Stiedl, Marianna Bugiani, Henner Koch, Maarten H P Kole, Ursula Boschert, Robert C Wykes, Huibert D Mansvelder, Marjo S van der Knaap, Rogier Min
OBJECTIVE: Loss of function of the astrocyte-specific protein MLC1 leads to the childhood onset leukodystrophy "megalencephalic leukoencephalopathy with subcortical cysts" (MLC). Studies on isolated cells show a role for MLC1 in astrocyte volume regulation and suggest that disturbed brain ion and water homeostasis is central to the disease. Excitability of neuronal networks is particularly sensitive to ion and water homeostasis. In line with this, reports of seizures and epilepsy in MLC patients exist...
February 21, 2018: Annals of Neurology
Oumama El Ezzi, Anthony S de Buys Roessingh, Michèle Bigorre, Guillaume Captier
BACKGROUND: Sebaceous nevus is a congenital malformation of the skin that usually occurs on the scalp or face. Syndromic forms do rarely exist with associated cerebral and ocular malformations. The skin lesions are pale at birth and become irregular by puberty. In the adult patient, tumors (usually benign) develop from sebaceous nevus. Their surgical excision during childhood can give a better result in terms of the definitive scar. OBJECTIVES: The aim of this study is to analyze our cases of syndromic sebaceous hamartoma, perform a review of the existing literature, and propose guidelines for the therapeutic plan...
February 16, 2018: International Journal of Dermatology
Tracy Liu, Raghu Lingam, Kate Lycett, Fiona K Mensah, Joshua Muller, Harriet Hiscock, Md Hamidul Huque, Melissa Wake
OBJECTIVE: To estimate prevalence and persistence of 19 common paediatric conditions from infancy to 14-15 years. DESIGN: Population-based prospective cohort study. SETTING: Australia. PARTICIPANTS: Parallel cohorts assessed biennially from 2004 to 2014 from ages 0-1 and 4-5 years to 10-11 and 14-15 years, respectively, in the Longitudinal Study of Australian Children. MAIN OUTCOME MEASURES: 19 health conditions: 17 parent-reported, 2 (overweight/obesity, obesity) directly assessed...
February 16, 2018: Archives of Disease in Childhood
Xiaotang Cai, Dan Yu, Yongmei Xie, Hui Zhou
RATIONALE: Argininemia is an autosomal recessive inherited disorder of the urea cycle. Because of its atypical symptoms in early age, diagnosis can be delayed until the typical chronic manifestations - including spastic diplegia, deterioration in cognitive function, and epilepsy - appear in later childhood. PATIENT CONCERNS: A Chinese boy initially presented with severe stunting and partial growth hormone deficiency (PGHD) at 3 years old and was initially treated with growth hormone replacement therapy...
February 2018: Medicine (Baltimore)
Martina Persson, Neda Razaz, Kristina Tedroff, K S Joseph, Sven Cnattingius
OBJECTIVE: To investigate associations between Apgar score at five and 10 minutes across the entire range of score values (from 0 to 10) and risks of childhood cerebral palsy or epilepsy, and to analyse the effect of changes in Apgar scores from five to 10 minutes after birth in infants born ≥37 completed weeks. DESIGN, SETTING, AND PARTICIPANTS: Population based cohort study in Sweden, including 1 213 470 non-malformed live singleton infants, born at term between 1999 and 2012...
February 7, 2018: BMJ: British Medical Journal
Ana M Gutierrez-Colina, Aimee W Smith, Constance A Mara, Avani C Modi
OBJECTIVE: The objectives of this study were to examine the continuity of adherence barriers across stages of development in pediatric epilepsy and to assess the differential influence of barriers on several important clinical outcomes from early childhood to young adulthood, including adherence, seizures, and health-related quality of life (HRQOL). METHOD: A developmentally representative sample of youth 2-25years with epilepsy was obtained by combining data from five different studies...
February 9, 2018: Epilepsy & Behavior: E&B
Phillip L Pearl
PURPOSE OF REVIEW: Epilepsy syndromes are an important clinical construct in pediatric epilepsy, as they encompass recognizable patterns seen in patients with epilepsies, whether of the more benign variety or associated with encephalopathy. RECENT FINDINGS: Syndromes may be organized by age of onset: neonatal, infantile, childhood, or adolescent. The assignment of a syndrome has specific implications for diagnosis, management, and prognostication. The 2010 revised classification of the epilepsies by the International League Against Epilepsy preserved the syndrome approach, while progress in genetics continues to advance our understanding of the pathophysiology and overlap of the epilepsy syndromes...
February 2018: Continuum: Lifelong Learning in Neurology
Kota Tachibana, Toshihisa Hamada, Hiroki Tsuchiya, Takashi Shibata, Kazuyasu Fujii, Katsuhiro Kobayashi, Keiji Iwatsuki
We report two rare cases of childhood epilepsy patients who developed ethosuximide-induced Stevens-Johnson syndrome (SJS). Unlike typical SJS, the initial eruption of both patients presented well-demarcated, infiltrating firm papules mainly on the cheeks and the extensor aspects of the arms (case 1), and multiple vesicles on the soles and oral aphthosis (case 2), which closely mimicked viral exanthema. We diagnosed both patients with ethosuximide-induced SJS, based on the dosing period and the positive results of drug-induced lymphocyte stimulation test...
February 11, 2018: Journal of Dermatology
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